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Huntington disease models and human neuropathology: similarities and differences

Huntington disease models and human neuropathology: similarities and differences

http://www.wikidata.org/entity/Q33760994

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Huntington's disease: a clinical review.Genetics and neuropathology of Huntington's disease Risk-taking and pathological gambling behavior in Huntington's disease Neural correlates of impaired emotion processing in manifest Huntington's disease.Differential vulnerability of neurons in Huntington's disease: the role of cell type-specific features.Differential putaminal morphology in Huntington's disease, frontotemporal dementia and Alzheimer's disease.Mitochondrial calcium uptake capacity as a therapeutic target in the R6/2 mouse model of Huntington's disease.Cholesterol defect is marked across multiple rodent models of Huntington's disease and is manifest in astrocytes.Movement disorders: role of imaging in diagnosis.Huntington's disease (HD): degeneration of select nuclei, widespread occurrence of neuronal nuclear and axonal inclusions in the brainstem.Drosophila Models of Huntington's Disease exhibit sleep abnormalities Basal ganglia contributions to motor control: a vigorous tutor Mapping cortico-striatal connectivity onto the cortical surface: a new tractography-based approach to study Huntington disease.Cysteine proteases bleomycin hydrolase and cathepsin Z mediate N-terminal proteolysis and toxicity of mutant huntingtin.Modulation of lipid peroxidation and mitochondrial function improves neuropathology in Huntington's disease mice Mutant huntingtin's interaction with mitochondrial protein Drp1 impairs mitochondrial biogenesis and causes defective axonal transport and synaptic degeneration in Huntington's disease.Clinical severity of Huntington's disease does not always correlate with neuropathologic stage.Neuropsychiatry and White Matter Microstructure in Huntington's Disease Astrocytes generated from patient induced pluripotent stem cells recapitulate features of Huntington's disease patient cells Early-onset sleep defects in Drosophila models of Huntington's disease reflect alterations of PKA/CREB signaling.Altered diffusion tensor imaging measurements in aged transgenic Huntington disease rats Chorea as a clinical feature of the basophilic inclusion body disease subtype of fused-in-sarcoma-associated frontotemporal lobar degeneration Post-mortem Findings in Huntington's Deep Brain Stimulation: A Moving Target Due to Atrophy.Quantitative Susceptibility Mapping Suggests Altered Brain Iron in Premanifest Huntington Disease.Widespread AAV1- and AAV2-mediated transgene expression in the nonhuman primate brain: implications for Huntington's disease.Drosophila melanogaster as a model organism of brain diseases Loss of corticostriatal and thalamostriatal synaptic terminals precedes striatal projection neuron pathology in heterozygous Q140 Huntington's disease mice.Dysregulation of coordinated neuronal firing patterns in striatum of freely behaving transgenic rats that model Huntington's disease scyllo-Inositol promotes robust mutant Huntingtin protein degradation.Suppressing aberrant GluN3A expression rescues synaptic and behavioral impairments in Huntington's disease models.Induced Pluripotent Stem Cells in Huntington's Disease: Disease Modeling and the Potential for Cell-Based Therapy.Molecular mechanisms and potential therapeutical targets in Huntington's disease.LXR agonists: new potential therapeutic drug for neurodegenerative diseases.The influence of cannabinoids on generic traits of neurodegeneration.Pathology and function of nuclear amyloid. Protein homeostasis matters.iPSC-based drug screening for Huntington's disease.Transneuronal propagation of mutant huntingtin contributes to non-cell autonomous pathology in neurons.Current Perspective of Stem Cell Therapy in Neurodegenerative and Metabolic Diseases.Huntington's disease (HD): the neuropathology of a multisystem neurodegenerative disorder of the human brain.Magnetization transfer MR imaging demonstrates degeneration of the subcortical and cortical gray matter in Huntington disease.

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Huntington disease models and human neuropathology: similarities and differences

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2007 nî lūn-bûn

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2007年の論文

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Huntington disease models and human neuropathology: similarities and differences

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Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation

Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases

Normal huntingtin function: an alternative approach to Huntington's disease

Analysis of a very large trinucleotide repeat in a patient with juvenile Huntington's disease

Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice

Neurological abnormalities in a knock-in mouse model of Huntington's disease

Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity

Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats

Huntingtin: an iron-regulated protein essential for normal nuclear and perinuclear organelles

Dramatic mutation instability in HD mouse striatum: does polyglutamine load contribute to cell-specific vulnerability in Huntington's disease?

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